Multiple myeloma (MM), also known as Kahler's disease or plasmacytoma, is a malignant disease of the bone marrow. It is characterized by the increased occurrence of so-called plasma cells.
Around four to six out of every 100,000 people are diagnosed with the disease each year. Multiple myeloma accounts for around one tenth of all blood cancers and around 1% of all cancers.
The exact causes of this malignant disease are largely unknown. In some cases, close contact with ionizing radiation from radioactive substances is probably the cause. Exposure to pesticides is also discussed as a possible cause.
Age is also one of the accepted risk factors for multiple myeloma. Most cases of the disease only occur after the age of 45. The average age of onset is as high as 70.
Other risk factors are
- being male,
- African-American roots and
- multiple myeloma in first-degree relatives.
The following are also being discussed as possible risk factors
- severe obesity and an unhealthy diet with a low proportion of fruit and vegetables, as well as
- diseases of the immune system such as AIDS or herpes zoster (shingles).
Plasma cells normally undergo a maturation process after coming into contact with a corresponding antigen. These antigens include, for example, viral or bacterial components. The plasma cells can no longer divide after this process. However, they can produce antibodies, i.e. antibodies against the antigens.
In contrast to these plasma cells, degenerated plasma cells, which are increasingly produced in the bone marrow in multiple myeloma, divide in an uncontrolled manner.
They also produce large quantities of paraproteins or free light chains. These are proteins that are very similar in structure to antibodies. However, they cannot perform any function in the immune system and are therefore useless.
Due to the strong proliferation of the degenerated plasma cells, the normal blood-forming cells of the bone marrow no longer have a place. They are displaced. The degenerated plasma cells also attack the bone substance.
Blood plasma is obtained by centrifuging blood. In multiple myeloma, the blood plasma cell count is too high © Fly_dragonfly | AdobeStock
The malignant disease develops gradually and only shows clear symptoms in later stages of the disease. Before that, many patients suffer from unspecific general symptoms such as feelings of weakness or weight loss.
Changes in the blood count
Due to the infiltration of the bone marrow and the associated displacement of healthy blood-forming cells, bone marrow function is reduced. The result is anemia, which can manifest itself in the following symptoms:
- Shortness of breath
- palpitations under stress
- headaches
- poor concentration
- Pallor
If not only the red blood cells but also the white blood cells (leukocytes) are reduced, this is also referred to as leukopenia. Leukocytes have the task of fighting off pathogens. If the number of leukocytes in the blood is greatly reduced, the patient is at increased risk of infection.
Like leukopenia, thrombocytopenia can also be a consequence of multiple myeloma. The lack of thrombocytes, the blood platelets, can result in an increased tendency to bleed. Patients suffer from frequent nosebleeds or develop bruises quickly.
The blood count can therefore show a lack of
- red blood cells,
- white blood cells and
- platelets
may show. In contrast, defective immunoglobulins are increasingly found in the blood. These can significantly increase the viscosity (viscosity) of the blood. The thickened blood serum leads to circulatory disorders in the small blood vessels. This disturbed microcirculation can manifest itself in the form of
- light-headedness,
- dizziness,
- visual disturbances or
- headaches
manifest itself.
Breakdown of the bone substance
The diseased plasma cells communicate with the osteoclasts and osteoblasts in various ways. These cells are responsible for bone metabolism and physiological bone remodeling. The plasma cells increasingly stimulate the bone-degrading osteoclasts and at the same time inhibit the activity of the bone-building osteoblasts. This results in the dissolution of bone tissue with bone pain and increased bone fractures.
A typical X-ray finding of multiple myeloma is the so-called shotgun skull, which is caused by many small osteolyses in the skull bone. If the vertebral bodies are affected by osteolysis, patients develop back pain at an early stage. Due to the advanced age of the patients, these are often misdiagnosed as degenerative age-related changes. It is often only after several attempts at therapy that a diagnosis is made using imaging procedures, which then leads to a diagnosis later on.
The increased breakdown of bone substance can lead to an excess of calcium in the blood. In medical terminology, this phenomenon is referred to as hypercalcemia.
In the kidneys, this leads to an increased loss of water and sodium. The so-called glomerular filtration rate of the renal corpuscles decreases. Ultimately, kidney failure occurs due to the deposition of light chains.
According to the current state of research, a cure is not possible. The aim of treatment is therefore to alleviate symptoms and prolong life.
As a rule,symptom-free patients do not need to be treated immediately. However, regular check-ups are recommended so that treatment can be initiated quickly if the first symptoms occur.
With
- Elevated calcium levels in the blood,
- renal insufficiency,
- anaemia and
- involvement of the bones
immediate treatment is required. General signs of illness such as night sweats or weight loss as well as increased blood viscosity are also indications for treatment.
Intensified therapy concepts are preferred for patients under 70 years of age. The standard treatment is high-dose therapy with an autologous blood stem cell transplant. Patients usually receive a combination therapy consisting of cytostatic drugs and cortisone preparations.
In an autologous stem cell transplant, doctors obtain stem cells from the patient's bone marrow. After chemotherapy, these are returned to the patient. In many cases, regeneration of blood formation can be achieved within a short period of time.
In rarer cases, stem cells from another donor are transplanted. This is an allogeneic stem cell transplant.
Patients over the age of 70 usually receive conventional chemotherapy. Even if the treatment was successful, there is a high risk of recurrence. This means that there is a higher probability of the disease recurring. Drugs such as bortezomib or thalidomide are intended to delay the relapse and are therefore used for maintenance therapy.
Patients are often given bisphosphonates to support them, which are intended to stem the loss of bone substance. They can also alleviate pain caused by osteolysis. Irradiation of the affected bones can also be carried out to relieve pain.